On the countdown...

Alana hasn't been well. We've been spending a lot of time at the doctors and I mean at A LOT of doctors. There has been many talks about "progression", "overall deteriorating condition" etc. We have been applying "band-aids" to her and that's all. While she has appeared good to a large degree, this has come via increased fluids, increased pain/neuropathy meds, more down time including sleeping, and more breakdowns. She is incredibly swollen due to the third spacing (google is your friend and her heart has been under strain from this. Her GI system has almost completely shut down to the point that none of her meds are really working for her anymore and as some of you might remember she's been battling a simple sinus infection since November.

With this being said, Saturday morning at 6am we will be on our way to Cincinnati Children's Hospital until at least the 20th. She is seeing EVERY specialty this time and having many, many, procedures, and surgeries. Some of the testing is extremely invasive and complicated but what we can learn from it might help prevent even more surgeries.

Some of the tests being done are colonic manometry as well as antro-duodenal manometry. These require a 3-day stay at the hospital. First day is clean out, 2nd day she will be put under and scopes done as well as her g-tube removed and a tube inserted in it's place instead. This tube will monitor the upper part of the stomach/guts while another tube inserted into the bottom will measure things from that end. These tubes cannot be disturbed so she will not be allowed out of bed, for any reason, until the testing is done the following day. She will also not be allowed to eat or drink during this time and will only be kept hydrated via her CVL. On the final day she will be given a 30 minute time frame to eat so we can view what happens to her stomach/guts. The purpose of this test is to help us determine is it her brain not sending signals? Is her gut not getting them? Is the gut getting them but not able to do anything about it? Where is the break in the function. This can help us prevent her from having surgery to 'help' her GI system. We are at a loss as to how to help her as it stands now, there isn't anything more we can do here with the testing available and even on a less evasive scale. 

She will be having sinus surgery done as well to try and stop these chronic sinus infections and facial pain. This will be on the 10th. She will be admitted due to how poorly she does with basic functions after the fact but Cincy Children's work way different than here, they don't believe in waking kids up and rushing the process of hurry up and get home. They let them wake up on their own and slowly and with Alana that helps, it gives her a chance to stabilize her body vs. the huge temperature spikes we see (92-105 degrees) as well as the tachy and bradycardia she has. I am very hopeful that this will result in improved tolerance to surgery. 

There is so much more but I'll work on updating the rest of that this week. 

Moving forward...

Things have been rather busy here. As anyone with children knows things change all the time. Birthdays come and go and then they come again before your ready for them. Noelani is now 18, how the time does fly. You should listen to your mother about that, amongst other things. Shaylin is turning 14 next week and has blossomed into such a kind and gentle young woman. Alana has remained Alana. This next batch of photos will show some of her latest journey with what happened her last surgery, placement of a central line.

Long time no see...

It's been longer than it should have. What can I say, a lot has changed, a lot hasn't. This is going to be a story through pictures. There will be jumps and skips through this and it'll take me a few days to get them all uploaded.

Why expect more...

So lets just copy and paste the beginning of the last post onto here. I was so tired yesterday that no, I didn't get a chance to post how the day went.

The hospital called on the way out the door to tell me that the scanner was down so they'd have to move it to today's fun filled agenda. She had the ultrasound of her of her diaphragm done though and it was normal so that's good news. She did fantastic with her PFT but I guess she wasn't consistent enough and it cannot be used so yea, nothing learned from that.

Today we saw Dr. Young and you know, I love her and she tries but to hear her say that there's nothing that she can think of to make things better right now is so frustrating. She is frustrated and concerned with what's going on with Alana and the fact that she's been on O2 full time for a year now and it's not making sense. Is it her lungs? Her muscles? Her heart? Her metabolic system? Anyway--we go to do the HRCT which we hope is going to show us something and yep, it cannot be done b/c Alana cannot breath how they want her too so therefore it has yet again been rescheduled. AGHHHHH!



On Friday when she has her scopes done they are going to go ahead and do that as well. We spent 6 hours at 4 appointments today and left with not much. Dr. Young just called a bit ago is still frustrated and I can hear it in her voice as much as she's telling me it. No one knows what to do with Alana and that's getting old. Very, very old. Tomorrow we meet with GI and go over those issues so who knows, maybe this won't be another why-are-we-here trip :-/

I know when we get home and all the information from her HRCT, sleep study, scopes, etc. come back we may have some answers but I have learned to not get my hopes up. They are not able to give us anymore on the mitochondrial front aside from saying she needs to go on the cocktail when we return home. sigh.



Will update tomorrow if there's anything good to share and for sure when she's okay after the procedures on Friday.

1 down...

So today we had the appointment with the neurologist and he was a good guy. Okay well that's all on that front. No seriously, there was nothing learned from that appointment as of right now. They do feel she needs to be put on the mito cocktail as soon as we return for a minimum of 6 months. He also told me, as I already knew, that a lot of children do not respond to the cocktail at all. They are going to add a shyt load of labs to her list for when she's under on Friday and be in touch while we are still here. The good news is though that he was kind and did appear interested in her case.

Tomorrow she will be having the ultrasound of her diaphragm and then her HRCT. Both important to see what's going on with these lungs of hers. It's scary and all that but needed to be done like yesterday. On Wed. we meet with Dr. Young again but before that she's having her first PFT done so we will be meeting with Dr. Young with all that information so that is a good thing fo-sur.



She was wired after sleeping on the plane yesterday night and didn't fall asleep till after 1 a.m. Cincy time and was a p.i.a. to wake up this morning. She's been moody all day but what else can you expect, it's a 4 hour time difference and it shows.

I will update tomorrow night unless something goes south.

We couldn't get the spoons to stick lol..we suck!

Tomorrow, tomorrow, I love ya tomorrow...

Tomorrow is the fun filled flight day. I will do regular updates while we are there, promise. Here is our appointment list so far (not in any particular order b/c I cannot remember what is where at the moment):

New visit with mitochondrial neurologist (no one is sure what he is going to eant done yet so we go in the morning with the thought that the rest of the day will be done running tests)
HRCT
Ultrasound of the diaphragm
Sleep study
PFT
Stress test
Pulmonary visit
Colonoscopy/EGD
GI visit
Labs (of course)

I think I am missing something but for the life of me cannot remember what it is at the moment. I still have a lot to do to get ready but didn't want to not post that we are indeed going and let you know a basics of what's what with appointments. I will update each night time permitting of course!

Very overdue...

So yes, it's been awhile my old friend. I know with you though I can leave you for awhile, have time to figure things out, and return and you won't judge; you won't hate.

Honestly I have come here, to this page many times and started this very entry and walked away from it. For the first time it all became to much to look at. To much pain, to much uncertainty, and mostly just to much. Today I will hit the publish button and I will stop hiding from the world.

First and foremost things with Alana are at an impasse of sorts. I am going to give you a bridged version of how things have been going as it will suffice. Since December she has had "pneumonia" every month. Her fevers have been out of control and shes had a significant turn in regards to her muscle strength. She falls...a lot. Her gait has not returned to her normal-learning-to-walk-been-walking-along-time pace. When she runs her feet and legs are spaced out and she's wobbly. She has taken to falling out of the blue and looks confused about it stating that her leg just didn't work right suddenly.

It's to the point that PT & OT are doing formal evaluations again, even though they were done not 6 months ago, to see where she's at now.


Her GI system has not been playing nice well either and we've already gone through 2 Mic-Key tubes due to balloons breaking. The feeds are still hit and miss and mostly miss. She is uncomfortable and cries a lot when we do them faster than 60 mL/hour. There has been no weight gain. She is still dependent on 3 med's to do what she needs to do and it's not easy on her stomach either way.

Due to the frequent issues with her lungs now she's been at 2 liters since December, up to 3 for PT & OT in order to maintain above 90. When she goes to school for a few hours it never fails that I get a call from the nurse telling me that she's at 89-90 and they had to have her rest and do some deep breathing for awhile in order to get her to 95. What should they do?


Things here have changed dramatically and anyone who tells you their other children and their marriage are "fine" and "have no issues" with having a chronically ill sibling/child are full of shyt. Shaylin resents everything about Alana's illness. Noelani tries to fix everything about her baby sister and just wants to hold her and make it better. Alana herself today asked me why I pick on her and do mean things to her like giving her a 2nd belly button and not making her lungs not hurt. I am sorry is all I can say to her. I am so sorry baby.



No matter how long I put off this post there's nothing I can do to make this a happy or easy to read. It's not easy for me to type, it's not easy for me to think about. There's so many things that are floating in my mind and while I've let you all into our family and our struggles with Alana, Shaylin and Noelani there are somethings that even I cannot wrap my head around and are going to have to remain unsaid for the moment. In time they will come out, trust me.

Before I go 2 things:

1. This is not a pity post. I don't want pity. My family does not need pity. Sometimes things that we say and feel are not meant to be taken as woes me but more along the lines of 'they have to come out of my head so I can move past them'.

2. We are headed back to Cincinnati on Sunday. My mom is coming with Alana and I and we will be there about 8 days if things go smoothly. I will update soon on all that's going to be done and who we are seeing. It has been a pushed trip, about a month in the making, due to her change in status.

Smile. We are at the end for now.